Creutzfeld Jakob Disease

Creutzfeld-Jakob Disease (CJD) was first described in Europe in 1920 and 1921, though it probably occurred prior to that and was not recognized. This disease occurs at a rate of about one case per million persons worldwide and generally affects only persons aged 65 years or more. It presents as a rapidly progressive dementia terminating in death roughly four to five months after symptom onset. CJD can have an incubation period of up to 20 years. Diagnosis is based on observation of clinically compatible symptoms, and a definitive diagnosis can only be determined postmortem through histopathologic examination of central nervous system biopsy specimens. As mentioned previously, most cases of CJD appear spontaneously (94,95).

In 1995 a new variant of CJD (nvCJD) was identified in the United Kingdom. Clinically, this variant was very similar to classic CJD but affected much younger persons, generally in their thirties. From 1995 through June 2002, a total of 124 human cases of vCJD were reported in the United Kingdom, 6 cases in France, and 1 case each in Ireland, Italy, and the United States. The case-patients from Ireland and the United States had each lived in the United Kingdom for more than 5 years during the UK BSE epidemic. The discovery of nvCJD following the BSE outbreak in the UK is very important because it appears that the agent causing BSE is capable of crossing the species barrier and causing illness in humans in the form of nvCJD (95,96).